Update in Cystic Fibrosis 2018

doi:10.1164/rccm.201902-0310UP

Review

. 2019 May 15;199(10):1188-1194.

doi: 10.1164/rccm.201902-0310UP.

Update in Cystic Fibrosis 2018

Bonnie W Ramsey^{1

2

3}, Gregory P Downey^{4

5

6

7

8}, Christopher H Goss^{9

3

10}

Affiliations

¹ 1 Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington.
² 2 Center for Clinical and Translational Research and.
³ 3 Division of Pediatric Pulmonology, Department of Pediatrics, and.
⁴ 4 Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine.
⁵ 5 Department of Pediatrics, and.
⁶ 6 Department of Biomedical Research, National Jewish Health, Denver, Colorado; and.
⁷ 7 Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, and.
⁸ 8 Department of Microbiology and Immunology, University of Colorado, Aurora, Colorado.
⁹ 10 Cystic Fibrosis Foundation Therapeutics Development Network Coordinating Center, Seattle Children's Research Institute, Seattle, Washington.
¹⁰ 9 Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Washington, Seattle, Washington.

PMID: 30917288
PMCID: PMC6519861
DOI: 10.1164/rccm.201902-0310UP

Review

Update in Cystic Fibrosis 2018

Bonnie W Ramsey et al. Am J Respir Crit Care Med. 2019.

. 2019 May 15;199(10):1188-1194.

doi: 10.1164/rccm.201902-0310UP.

Authors

Bonnie W Ramsey^{1

2

3}, Gregory P Downey^{4

5

6

7

8}, Christopher H Goss^{9

3

10}

Affiliations

¹ 1 Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington.
² 2 Center for Clinical and Translational Research and.
³ 3 Division of Pediatric Pulmonology, Department of Pediatrics, and.
⁴ 4 Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine.
⁵ 5 Department of Pediatrics, and.
⁶ 6 Department of Biomedical Research, National Jewish Health, Denver, Colorado; and.
⁷ 7 Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, and.
⁸ 8 Department of Microbiology and Immunology, University of Colorado, Aurora, Colorado.
⁹ 10 Cystic Fibrosis Foundation Therapeutics Development Network Coordinating Center, Seattle Children's Research Institute, Seattle, Washington.
¹⁰ 9 Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Washington, Seattle, Washington.

PMID: 30917288
PMCID: PMC6519861
DOI: 10.1164/rccm.201902-0310UP

No abstract available

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References

1. Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, et al. VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011;365:1663–1672. - PMC - PubMed
1. Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, et al. TRAFFIC Study Group; TRANSPORT Study Group. Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015;373:220–231. - PMC - PubMed
1. Donaldson SH, Pilewski JM, Griese M, Cooke J, Viswanathan L, Tullis E, et al. VX11-661-101 Study Group. Tezacaftor/ivacaftor in subjects with cystic fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR. Am J Respir Crit Care Med. 2018;197:214–224. - PMC - PubMed
1. Rowe SM, Daines C, Ringshausen FC, Kerem E, Wilson J, Tullis E, et al. Tezacaftor-ivacaftor in residual-function heterozygotes with cystic fibrosis. N Engl J Med. 2017;377:2024–2035. - PMC - PubMed
1. Keating D, Marigowda G, Burr L, Daines C, Mall MA, McKone EF, et al. VX16-445-001 Study Group. VX-445-tezacaftor-ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles. N Engl J Med. 2018;379:1612–1620. - PMC - PubMed

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[1] Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, et al. VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011;365:1663–1672. - PMC - PubMed

[2] Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, et al. VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011;365:1663–1672. - PMC - PubMed

[3] Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, et al. TRAFFIC Study Group; TRANSPORT Study Group. Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015;373:220–231. - PMC - PubMed

[4] Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, et al. TRAFFIC Study Group; TRANSPORT Study Group. Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015;373:220–231. - PMC - PubMed

[5] Donaldson SH, Pilewski JM, Griese M, Cooke J, Viswanathan L, Tullis E, et al. VX11-661-101 Study Group. Tezacaftor/ivacaftor in subjects with cystic fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR. Am J Respir Crit Care Med. 2018;197:214–224. - PMC - PubMed

[6] Donaldson SH, Pilewski JM, Griese M, Cooke J, Viswanathan L, Tullis E, et al. VX11-661-101 Study Group. Tezacaftor/ivacaftor in subjects with cystic fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR. Am J Respir Crit Care Med. 2018;197:214–224. - PMC - PubMed

[7] Rowe SM, Daines C, Ringshausen FC, Kerem E, Wilson J, Tullis E, et al. Tezacaftor-ivacaftor in residual-function heterozygotes with cystic fibrosis. N Engl J Med. 2017;377:2024–2035. - PMC - PubMed

[8] Rowe SM, Daines C, Ringshausen FC, Kerem E, Wilson J, Tullis E, et al. Tezacaftor-ivacaftor in residual-function heterozygotes with cystic fibrosis. N Engl J Med. 2017;377:2024–2035. - PMC - PubMed

[9] Keating D, Marigowda G, Burr L, Daines C, Mall MA, McKone EF, et al. VX16-445-001 Study Group. VX-445-tezacaftor-ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles. N Engl J Med. 2018;379:1612–1620. - PMC - PubMed

[10] Keating D, Marigowda G, Burr L, Daines C, Mall MA, McKone EF, et al. VX16-445-001 Study Group. VX-445-tezacaftor-ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles. N Engl J Med. 2018;379:1612–1620. - PMC - PubMed

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Update in Cystic Fibrosis 2018

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Update in Cystic Fibrosis 2018

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